Endocrine Abstracts

Introduction: Ectopic Cushing’s syndrome constitutes the second most common paraneoplastic syndrome and has been seen in 1-5 % of small cell lung cancers. It has a poor prognosis and can present with life-threatening complications.

Case: We present a 66-year-old lady who attended with peripheral oedema, bruising and visual blurring. Past medical history included bronchiectasis, oesophageal web, ischemic colitis, Ehlers Danlos syndrome, and hypoxic brain injury. For 2 years prior to presentation, she had used octreotide to manage diarrhoea associated with treatment for ischaemic colitis. She was also on long-term anticoagulation given history of thrombosis. On admission she was hypokalaemia (2.7 mmol/l) and hypertensive (171/85). Clinical examination was consistent with cushingoid features. Investigations showed cortisol >1750 nmol/l unsuppressed following dexamethasone with elevated ACTH 345ng/l and high urinary free cortisol: 20568 nmol/24 hour. She started metyrapone 250 mg QDS. Treatment was complicated by E coli urinary sepsis and then hypertensive crisis with posterior reversible encephalopathy (PRES). She and her family had previously refused level 2/3 care. MRI Pituitary showed no adenoma and CTPA revealed bilateral pulmonary nodules with a left upper lobe mass. Despite metyrapone 1.5g QDS alongside ketoconazole she remained hypercortisolemic and deteriorated. Palliative care was then offered.

Conclusion: Rapidity and severity of symptoms associated with hypercortisolism can be challenging to manage. Octreotide may have delayed her clinical presentation. Although the main causes of death with active Cushing’s are infections, cardiovascular disease, and venous thromboembolism, it is important to remain mindful of the rarer complications. PRES is a serious complication associated with severe hypercortisolism which is reversible with aggressive treatment but usually associated with poor outcomes. Through this case we highlight the many options that can be used to treat active Cushing’s syndrome alongside some of their limitations.